Original Article
Orbital Preservation in Patients with Esthesioneuroblastoma Marc W. Herr1
Stacey T. Gray1
Audrey B. Erman2
1 Department of Otology and Laryngology, Massachusetts Eye and Ear
Infirmary/Massachusetts General Hospital Cranial Base Center, Boston, Massachusetts, USA 2 Department of Otolaryngology–Head and Neck Surgery, University of Arizona Cancer Center, Tucson, Arizona, USA 3 Department of Neurosurgery, Pappas Center for Neuro-oncology, Massachusetts General Hospital, Boston, Massachusetts, USA
William T. Curry3
Daniel G. Deschler1
Derrick T. Lin1
Address for correspondence Marc W. Herr, MD, Department of Otology and Laryngology, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA (e-mail: [email protected]).
J Neurol Surg B 2013;74:142–145.
Abstract
Keywords
► esthesioneuroblastoma ► anterior craniofacial resection ► orbital preservation ► skull base
Objectives Surgical resection in addition to adjuvant radiation with or without chemotherapy is the mainstay of treatment for esthesioneuroblastoma (ENB). However, management of patients with orbital involvement remains controversial. Historically, orbital exenteration has been advocated when there is evidence of periorbital invasion. Recently, the indications for orbital exenteration have become more selective and orbital preservation has been advocated. We report our experience with anterior craniofacial resection and orbital preservation in patients with ENB. Design Retrospective review of all patients diagnosed with esthesioneuroblastoma who underwent traditional open anterior craniofacial resection at the Massachusetts General Hospital/Massachusetts Eye and Ear Infirmary Cranial Base Center from 1997 to 2008. Results Sixteen patients were identified with a mean follow-up of 76 months. All patients underwent anterior craniofacial resection via an open approach and adjuvant proton beam radiation. Six of the 16 patients had evidence of either periorbital or lacrimal sac involvement at the time of surgery. All of these patients underwent periorbital resection to negative histologic margins with preservation of the orbit. Conclusion In our study, patients with ENB and periorbital invasion—who were treated with anterior craniofacial resection and periorbital resection with orbital preservation— had no evidence of decreased survival. In all patients, negative histologic margins of the periorbital resection were achieved.
Introduction First described by Berger et al in 1924, esthesioneuroblastoma (ENB) is a rare malignant tumor of the sinonasal vault believed to arise from the olfactory epithelium.1 Historically there have been several proposed strategies for management of ENB. In general, surgical resection with adjuvant postoper-
received June 23, 2012 accepted December 17, 2012 published online March 13, 2013
ative radiation therapy, with or without chemotherapy, has been the mainstay of treatment.2–8 Because of the typical location of ENB within the superior nasal vault and sinonasal cavity, close approximation or frank involvement of the orbit is often seen at the time of initial assessment. Typically, surgical treatment for sinonasal tumors involving the periorbita or orbit included oncologic
© 2013 Georg Thieme Verlag KG Stuttgart · New York
DOI http://dx.doi.org/ 10.1055/s-0033-1338259. ISSN 2193-6331.
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
142
Orbital Preservation in Esthesioneuroblastoma
Materials and Methods The medical records of all patients diagnosed with ENB who underwent traditional anterior craniofacial resection at the Massachusetts General Hospital/Massachusetts Eye and Ear Infirmary Cranial Base Center between 1997 and 2008 were reviewed. Institutional review board (IRB) approval was obtained for this study. For the purpose of this study, orbital involvement was defined as histologic involvement of the periorbita. Sixteen patients were identified and included in the study. Preoperative clinical data, imaging studies, operative notes, pathology reports, and follow-up medical records were reviewed for each patient. The patients were then divided into two categories: Group A, whose tumors did not invade the periorbita, and Group B, whose tumors did
143
invade the periorbita. The records were then examined to determine the long-term treatment results. The primary end point of the study was overall 5-year survival. Surgical resection included traditional anterior craniofacial resection for all patients. During surgery the periorbita was inspected. If there was evidence of periorbital involvement, resection was performed with frozen sections utilized to confirm negative margins. All patients received postoperative proton beam radiation therapy. Seven patients received adjuvant chemotherapy.
Results Our series included sixteen patients—eight women and eight men. Their mean age at diagnosis was 47 years, ranging from 11 to 77 years. Mean follow-up time was 76 months. Ten patients were found to have no periorbital involvement (Group A )(►Table 1) and six patients were found to have evidence of periorbital involvement (Group B) (►Table 2). Four patients developed recurrences an average of 57.8 months after diagnosis. Two of these patients were in Group A and two were in Group B. In Group A, Patient 3 underwent CFR with postoperative proton beam radiation. He recurred locally at the edge of his frontal craniotomy bone flap 72 months after diagnosis. Recurrent tumor was managed with surgical excision and re-irradiation. He is currently alive without disease 125 months after his initial treatment. Patient 7 developed distant metastases to the brain and spine, which were managed with palliative chemotherapy. This patient ultimately succumbed to disease 31 months after diagnosis. In Group B, patient 2 was treated with neoadjuvant chemotherapy, followed by CFR and mixed photon/proton
Table 1 Group A. Treatment and Outcomes: No Periorbital Involvement Patient
Treatment
Total RT (Gy)
Chemo
Recurrence (months)
Final status
Total follow-up time (months)
1
CFR þ proton/photon RT
66
None
No
NED
85
2
CFR þ proton/photon RT
59.4
None
No
NED
106
3
CFR þ proton/photon RT
59.2
None
Yes (72)
NED
125
4
CFR þ proton RT
54
None
No
NED
113
5
CFR þ proton RT
70
None
No
NED
75
6
Endoscopic resection (partial) ! CFR þ proton RT
54
None
No
NED
82
7
CT (NR) ! CFR þ proton/photon RT þ CT
67.2
2 cycles cisplatin, etoposide ! NR ! 2 cycles carboplatin, etoposide
Yes (16)
DOD
31
8
CFR þ proton RT þ CT
68
4 cycles carboplatin
No
NED
30
9
CFR þ proton RT
70
None
No
NED
65
10
CFR þ proton RT
66
None
No
NED
35
Abbreviations: AWD, alive with disease; CFR, craniofacial resection; CT, chemotherapy; DOD, dead of disease; NED, no evidence of disease; NR, no response; RT, radiotherapy. Journal of Neurological Surgery—Part B
Vol. 74
No. B3/2013
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
resection and orbital exenteration.9 More recently, the indications for orbital exenteration have become more selective, and orbital preservation has been advocated in a variety of settings.10–12 The current treatment regimen for patients with ENB and orbital involvement at the Massachusetts General Hospital/ Massachusetts Eye and Ear Infirmary Cranial Base Center consists of craniofacial resection (CFR) with orbital preservation followed by proton beam radiation therapy with or without chemotherapy.13 Previous review of this protocol demonstrated 5-year disease-free and overall survival (OS) rates of 90% and 87.5%, respectively—which compares favorably with those of other institutions.4,6,13,14 The goal of the present study is to review our experience with orbital preservation in patients with ENB and periorbital involvement who underwent treatment with an intent to cure.
Herr et al.
Orbital Preservation in Esthesioneuroblastoma
Herr et al.
Table 2 Group B. Treatment and Outcomes: Positive Periorbital Involvement Patient
Treatment
Total RT (Gy)
Chemo
Recurrence (months)
Final status
Total follow-up time (months)
1
CFR þ proton/photon RT þ CT
70.2
4 cycles cisplatin, etoposide
No
NED
77
2
CT (NR) ! CFR þ proton/photon RT
67
2 cycles cisplatin, etoposide
Yes (82)
AWD
176
3
CT (NR) ! CFR þ proton RT
60
2 cycles cisplatin, etoposide
Yes (61 & 143)
AWD
146
4
CFR þ proton RT þ CT
60
4 cycles cisplatin, etoposide
No
NED
33
5
CFR þ proton RT þ CT
60
4 cycles cisplatin, etoposide
No
NED
38
6
CFR þ proton RT
66
None
No
NED
32
Abbreviations: AWD, alive with disease; CFR, craniofacial resection; CT, chemotherapy; DOD, dead of disease; NED, no evidence of disease; NR, no response; RT, radiotherapy.
irradiation. He recurred both regionally and distantly and was managed with surgical resection, chemotherapy, and reirradiation. He is alive with probable disease 176 months after initial treatment. Patient 3 was also initially treated with chemotherapy followed by CFR and proton beam radiation. She recurred regionally 61 months after diagnosis and was managed with a parotidectomy and neck dissection. At 143 months following her diagnosis, she was found to have a second recurrence in the nasopharynx and cervical lymph nodes. She is currently undergoing proton beam radiation and concomitant weekly chemotherapy with carboplatin and Taxol (Bristol-Myers Squibb, New York, New York, USA). Kaplan-Meier survival curves were constructed for 5-year OS rates (►Fig. 1). The calculated 5-year OS was 87.5%. There was no statistically significant difference in overall between Group A and Group B (p ¼ 0.4).
Discussion Since the early 1970s, several guidelines for orbital preservation in the setting of sinonasal malignancies have been proposed based on the degree of tumor involvement of the bony orbital wall and orbital structures.10–19 The evolution of treatment has been toward orbital preservation. Sisson in 1970 was one of the first to advocate orbital preservation, recommending preoperative radiation therapy followed by intraoperative evaluation.10 Harrison and Som later reported their experiences, with each recommending orbital exenter-
Fig. 1 Overall survival. Journal of Neurological Surgery—Part B
Vol. 74
No. B3/2013
ation only in the setting of bone erosion determined at the time of surgical resection.11,12 In 1980, Weymuller et al reported that their series of patients—whose orbit was preserved in the face of bone erosion—had a similar prognosis to those who were treated with orbital exenteration.15 In 1988, Perry et al examined 41 patients with malignancies that abutted the orbital walls, 26 of which eroded through bone.16 In this study, if the tumor could be peeled from the periorbita, the eye was saved; if the periorbita was only minimally involved, it was locally resected with frozen-section control. Using this algorithm, they reported that preservation of the orbit did not alter local control or OS. These findings were further supported by Imola et al in 2002.19 They reported on 66 patients undergoing treatment for sinonasal malignancy encroaching the orbit. Their indications for orbital exenteration included (1) involvement of the orbital apex, (2) nonresectable full-thickness invasion through periorbita into retrobulbar fat, (3) extension into the extraocular eye muscles, (4) invasion of the bulbar conjunctiva or sclera, and (5) lid involvement beyond a reasonable hope for reconstruction. The authors concluded that selective orbital preservation is oncologically safe and that consideration should be given to rigid orbital reconstruction in larger defects resulting from subtotal or total orbital floor resection or resections involving two or more orbital floors. Unlike previous reports, our series reviewed a single disease entity. None of our 16 patients would have fulfilled Imola’s criteria for orbital exenteration. The average time to recurrence of our four patients was 57.8 months, which is consistent with the late recurrences noted in the literature and reinforces the need for continued long-term follow-up. Of these four recurrences, two had gross periorbital invasion at the time of surgery and two did not. Of note, all three of the patients who underwent initial chemotherapy instead of upfront surgery recurred, whereas only one of 13 patients who underwent initial CFR recurred. Overall, there was no statistically significant difference between Group A and Group B. In fact, the only death in our series occurred in a patient without periorbital involvement.
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
144
Orbital Preservation in Esthesioneuroblastoma
Conclusion In our series of patients treated for ENB, we believe that sparing the orbit in the face of gross or microscopic periorbital involvement is oncologically reasonable. Our experience with these 16 patients, 6 of whom had invasion of the periorbita, supports preservation of the orbit with resection of the periorbita utilizing frozen section control followed by proton beam radiotherapy with or without chemotherapy.
7 Ward PD, Heth JA, Thompson BG, Marentette LJ. Esthesioneuro-
8
9
10
11 12 13
References 1 Berger L, Luc R, Richard D. L’esthésioneuroépithéliome olfactif. Bull 2
3 4
5
6
Assoc Fr Etud Cancer 1924;13:410–421 Bhattacharyya N, Thornton AF, Joseph MP, Goodman ML, Amrein PC. Successful treatment of esthesioneuroblastoma and neuroendocrine carcinoma with combined chemotherapy and proton radiation. Results in 9 cases. Arch Otolaryngol Head Neck Surg 1997;123(1):34–40 Dulguerov P, Allal AS, Calcaterra TC. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2001;2(11):683–690 Levine PA, Gallagher R, Cantrell RW. Esthesioneuroblastoma: reflections of a 21-year experience. Laryngoscope 1999;109(10): 1539–1543 Nishimura H, Ogino T, Kawashima M, et al. Proton-beam therapy for olfactory neuroblastoma. Int J Radiat Oncol Biol Phys 2007; 68(3):758–762 Simon JH, Zhen W, McCulloch TM, et al. Esthesioneuroblastoma: the University of Iowa experience 1978-1998. Laryngoscope 2001; 111(3):488–493
145
14 15
16
17
18
19
blastoma: results and outcomes of a single institution’s experience. Skull Base 2009;19(2):133–140 Resto VA, Eisele DW, Forastiere A, Zahurak M, Lee DJ, Westra WH. Esthesioneuroblastoma: the Johns Hopkins experience. Head Neck 2000;22(6):550–558 Ketcham AS, Chretien PB, Van Buren JM, Hoye RC, Beazley RM, Herdt JR. The ethmoid sinuses: a re-evaluation of surgical resection. Am J Surg 1973;126(4):469–476 Sisson GA. Symposium: 3. Treatment of malignancies of paranasal sinuses. Discussion and summary. Laryngoscope 1970;80(6): 945–953 Harrison DFN. Problems in surgical management of neoplasms arising in the paranasal sinuses. J Laryngol Otol 1976;90(1):69–74 Som ML. Surgical management of carcinoma of the maxilla. Arch Otolaryngol 1974;99(4):270–273 Nichols AC, Chan AW, Curry WT, Barker FG, Deschler DG, Lin DT. Esthesioneuroblastoma: the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy. Skull Base 2008;18(5):327–337 Lund VJ, Howard D, Wei W, Spittle M. Olfactory neuroblastoma: past, present, and future? Laryngoscope 2003;113(3):502–507 Weymuller EA Jr, Reardon EJ, Nash D. A comparison of treatment modalities in carcinoma of the maxillary antrum. Arch Otolaryngol 1980;106(10):625–629 Perry C, Levine PA, Williamson BR, Cantrell RW. Preservation of the eye in paranasal sinus cancer surgery. Arch Otolaryngol Head Neck Surg 1988;114(6):632–634 McCary SW, Levine PA, Cantrell RW. Preservation of the eye in the treatment of sinonasal malignant neoplasms with orbital involvment. A confirmation of the original treatise. Arch Otolaryngol Head Neck Surg 1996; 122(6):657–659 Essig GF, Newman SA, Levine PA. Sparing the eye in craniofacial surgery for superior nasal vault malignant neoplasms: analysis of benefit. Arch Facial Plast Surg 2007;9(6):406–411 Imola MJ, Schramm VL Jr. Orbital preservation in surgical management of sinonasal malignancy. Laryngoscope 2002;112(8 Pt 1): 1357–1365
Journal of Neurological Surgery—Part B
Vol. 74
No. B3/2013
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
All patients in our series had preserved vision and extraocular motion after completion of treatment. The incidence of reported epiphora was equal in both groups.
Herr et al.
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Orbital Preservation in Patients with Esthesioneuroblastoma Marc W. Herr1
Stacey T. Gray1
Audrey B. Erman2
1 Department of Otology and Laryngology, Massachusetts Eye and Ear
Infirmary/Massachusetts General Hospital Cranial Base Center, Boston, Massachusetts, USA 2 Department of Otolaryngology–Head and Neck Surgery, University of Arizona Cancer Center, Tucson, Arizona, USA 3 Department of Neurosurgery, Pappas Center for Neuro-oncology, Massachusetts General Hospital, Boston, Massachusetts, USA
William T. Curry3
Daniel G. Deschler1
Derrick T. Lin1
Address for correspondence Marc W. Herr, MD, Department of Otology and Laryngology, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA (e-mail: [email protected]).
J Neurol Surg B 2013;74:142–145.
Abstract
Keywords
► esthesioneuroblastoma ► anterior craniofacial resection ► orbital preservation ► skull base
Objectives Surgical resection in addition to adjuvant radiation with or without chemotherapy is the mainstay of treatment for esthesioneuroblastoma (ENB). However, management of patients with orbital involvement remains controversial. Historically, orbital exenteration has been advocated when there is evidence of periorbital invasion. Recently, the indications for orbital exenteration have become more selective and orbital preservation has been advocated. We report our experience with anterior craniofacial resection and orbital preservation in patients with ENB. Design Retrospective review of all patients diagnosed with esthesioneuroblastoma who underwent traditional open anterior craniofacial resection at the Massachusetts General Hospital/Massachusetts Eye and Ear Infirmary Cranial Base Center from 1997 to 2008. Results Sixteen patients were identified with a mean follow-up of 76 months. All patients underwent anterior craniofacial resection via an open approach and adjuvant proton beam radiation. Six of the 16 patients had evidence of either periorbital or lacrimal sac involvement at the time of surgery. All of these patients underwent periorbital resection to negative histologic margins with preservation of the orbit. Conclusion In our study, patients with ENB and periorbital invasion—who were treated with anterior craniofacial resection and periorbital resection with orbital preservation— had no evidence of decreased survival. In all patients, negative histologic margins of the periorbital resection were achieved.
Introduction First described by Berger et al in 1924, esthesioneuroblastoma (ENB) is a rare malignant tumor of the sinonasal vault believed to arise from the olfactory epithelium.1 Historically there have been several proposed strategies for management of ENB. In general, surgical resection with adjuvant postoper-
received June 23, 2012 accepted December 17, 2012 published online March 13, 2013
ative radiation therapy, with or without chemotherapy, has been the mainstay of treatment.2–8 Because of the typical location of ENB within the superior nasal vault and sinonasal cavity, close approximation or frank involvement of the orbit is often seen at the time of initial assessment. Typically, surgical treatment for sinonasal tumors involving the periorbita or orbit included oncologic
© 2013 Georg Thieme Verlag KG Stuttgart · New York
DOI http://dx.doi.org/ 10.1055/s-0033-1338259. ISSN 2193-6331.
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
142
Orbital Preservation in Esthesioneuroblastoma
Materials and Methods The medical records of all patients diagnosed with ENB who underwent traditional anterior craniofacial resection at the Massachusetts General Hospital/Massachusetts Eye and Ear Infirmary Cranial Base Center between 1997 and 2008 were reviewed. Institutional review board (IRB) approval was obtained for this study. For the purpose of this study, orbital involvement was defined as histologic involvement of the periorbita. Sixteen patients were identified and included in the study. Preoperative clinical data, imaging studies, operative notes, pathology reports, and follow-up medical records were reviewed for each patient. The patients were then divided into two categories: Group A, whose tumors did not invade the periorbita, and Group B, whose tumors did
143
invade the periorbita. The records were then examined to determine the long-term treatment results. The primary end point of the study was overall 5-year survival. Surgical resection included traditional anterior craniofacial resection for all patients. During surgery the periorbita was inspected. If there was evidence of periorbital involvement, resection was performed with frozen sections utilized to confirm negative margins. All patients received postoperative proton beam radiation therapy. Seven patients received adjuvant chemotherapy.
Results Our series included sixteen patients—eight women and eight men. Their mean age at diagnosis was 47 years, ranging from 11 to 77 years. Mean follow-up time was 76 months. Ten patients were found to have no periorbital involvement (Group A )(►Table 1) and six patients were found to have evidence of periorbital involvement (Group B) (►Table 2). Four patients developed recurrences an average of 57.8 months after diagnosis. Two of these patients were in Group A and two were in Group B. In Group A, Patient 3 underwent CFR with postoperative proton beam radiation. He recurred locally at the edge of his frontal craniotomy bone flap 72 months after diagnosis. Recurrent tumor was managed with surgical excision and re-irradiation. He is currently alive without disease 125 months after his initial treatment. Patient 7 developed distant metastases to the brain and spine, which were managed with palliative chemotherapy. This patient ultimately succumbed to disease 31 months after diagnosis. In Group B, patient 2 was treated with neoadjuvant chemotherapy, followed by CFR and mixed photon/proton
Table 1 Group A. Treatment and Outcomes: No Periorbital Involvement Patient
Treatment
Total RT (Gy)
Chemo
Recurrence (months)
Final status
Total follow-up time (months)
1
CFR þ proton/photon RT
66
None
No
NED
85
2
CFR þ proton/photon RT
59.4
None
No
NED
106
3
CFR þ proton/photon RT
59.2
None
Yes (72)
NED
125
4
CFR þ proton RT
54
None
No
NED
113
5
CFR þ proton RT
70
None
No
NED
75
6
Endoscopic resection (partial) ! CFR þ proton RT
54
None
No
NED
82
7
CT (NR) ! CFR þ proton/photon RT þ CT
67.2
2 cycles cisplatin, etoposide ! NR ! 2 cycles carboplatin, etoposide
Yes (16)
DOD
31
8
CFR þ proton RT þ CT
68
4 cycles carboplatin
No
NED
30
9
CFR þ proton RT
70
None
No
NED
65
10
CFR þ proton RT
66
None
No
NED
35
Abbreviations: AWD, alive with disease; CFR, craniofacial resection; CT, chemotherapy; DOD, dead of disease; NED, no evidence of disease; NR, no response; RT, radiotherapy. Journal of Neurological Surgery—Part B
Vol. 74
No. B3/2013
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
resection and orbital exenteration.9 More recently, the indications for orbital exenteration have become more selective, and orbital preservation has been advocated in a variety of settings.10–12 The current treatment regimen for patients with ENB and orbital involvement at the Massachusetts General Hospital/ Massachusetts Eye and Ear Infirmary Cranial Base Center consists of craniofacial resection (CFR) with orbital preservation followed by proton beam radiation therapy with or without chemotherapy.13 Previous review of this protocol demonstrated 5-year disease-free and overall survival (OS) rates of 90% and 87.5%, respectively—which compares favorably with those of other institutions.4,6,13,14 The goal of the present study is to review our experience with orbital preservation in patients with ENB and periorbital involvement who underwent treatment with an intent to cure.
Herr et al.
Orbital Preservation in Esthesioneuroblastoma
Herr et al.
Table 2 Group B. Treatment and Outcomes: Positive Periorbital Involvement Patient
Treatment
Total RT (Gy)
Chemo
Recurrence (months)
Final status
Total follow-up time (months)
1
CFR þ proton/photon RT þ CT
70.2
4 cycles cisplatin, etoposide
No
NED
77
2
CT (NR) ! CFR þ proton/photon RT
67
2 cycles cisplatin, etoposide
Yes (82)
AWD
176
3
CT (NR) ! CFR þ proton RT
60
2 cycles cisplatin, etoposide
Yes (61 & 143)
AWD
146
4
CFR þ proton RT þ CT
60
4 cycles cisplatin, etoposide
No
NED
33
5
CFR þ proton RT þ CT
60
4 cycles cisplatin, etoposide
No
NED
38
6
CFR þ proton RT
66
None
No
NED
32
Abbreviations: AWD, alive with disease; CFR, craniofacial resection; CT, chemotherapy; DOD, dead of disease; NED, no evidence of disease; NR, no response; RT, radiotherapy.
irradiation. He recurred both regionally and distantly and was managed with surgical resection, chemotherapy, and reirradiation. He is alive with probable disease 176 months after initial treatment. Patient 3 was also initially treated with chemotherapy followed by CFR and proton beam radiation. She recurred regionally 61 months after diagnosis and was managed with a parotidectomy and neck dissection. At 143 months following her diagnosis, she was found to have a second recurrence in the nasopharynx and cervical lymph nodes. She is currently undergoing proton beam radiation and concomitant weekly chemotherapy with carboplatin and Taxol (Bristol-Myers Squibb, New York, New York, USA). Kaplan-Meier survival curves were constructed for 5-year OS rates (►Fig. 1). The calculated 5-year OS was 87.5%. There was no statistically significant difference in overall between Group A and Group B (p ¼ 0.4).
Discussion Since the early 1970s, several guidelines for orbital preservation in the setting of sinonasal malignancies have been proposed based on the degree of tumor involvement of the bony orbital wall and orbital structures.10–19 The evolution of treatment has been toward orbital preservation. Sisson in 1970 was one of the first to advocate orbital preservation, recommending preoperative radiation therapy followed by intraoperative evaluation.10 Harrison and Som later reported their experiences, with each recommending orbital exenter-
Fig. 1 Overall survival. Journal of Neurological Surgery—Part B
Vol. 74
No. B3/2013
ation only in the setting of bone erosion determined at the time of surgical resection.11,12 In 1980, Weymuller et al reported that their series of patients—whose orbit was preserved in the face of bone erosion—had a similar prognosis to those who were treated with orbital exenteration.15 In 1988, Perry et al examined 41 patients with malignancies that abutted the orbital walls, 26 of which eroded through bone.16 In this study, if the tumor could be peeled from the periorbita, the eye was saved; if the periorbita was only minimally involved, it was locally resected with frozen-section control. Using this algorithm, they reported that preservation of the orbit did not alter local control or OS. These findings were further supported by Imola et al in 2002.19 They reported on 66 patients undergoing treatment for sinonasal malignancy encroaching the orbit. Their indications for orbital exenteration included (1) involvement of the orbital apex, (2) nonresectable full-thickness invasion through periorbita into retrobulbar fat, (3) extension into the extraocular eye muscles, (4) invasion of the bulbar conjunctiva or sclera, and (5) lid involvement beyond a reasonable hope for reconstruction. The authors concluded that selective orbital preservation is oncologically safe and that consideration should be given to rigid orbital reconstruction in larger defects resulting from subtotal or total orbital floor resection or resections involving two or more orbital floors. Unlike previous reports, our series reviewed a single disease entity. None of our 16 patients would have fulfilled Imola’s criteria for orbital exenteration. The average time to recurrence of our four patients was 57.8 months, which is consistent with the late recurrences noted in the literature and reinforces the need for continued long-term follow-up. Of these four recurrences, two had gross periorbital invasion at the time of surgery and two did not. Of note, all three of the patients who underwent initial chemotherapy instead of upfront surgery recurred, whereas only one of 13 patients who underwent initial CFR recurred. Overall, there was no statistically significant difference between Group A and Group B. In fact, the only death in our series occurred in a patient without periorbital involvement.
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
144
Orbital Preservation in Esthesioneuroblastoma
Conclusion In our series of patients treated for ENB, we believe that sparing the orbit in the face of gross or microscopic periorbital involvement is oncologically reasonable. Our experience with these 16 patients, 6 of whom had invasion of the periorbita, supports preservation of the orbit with resection of the periorbita utilizing frozen section control followed by proton beam radiotherapy with or without chemotherapy.
7 Ward PD, Heth JA, Thompson BG, Marentette LJ. Esthesioneuro-
8
9
10
11 12 13
References 1 Berger L, Luc R, Richard D. L’esthésioneuroépithéliome olfactif. Bull 2
3 4
5
6
Assoc Fr Etud Cancer 1924;13:410–421 Bhattacharyya N, Thornton AF, Joseph MP, Goodman ML, Amrein PC. Successful treatment of esthesioneuroblastoma and neuroendocrine carcinoma with combined chemotherapy and proton radiation. Results in 9 cases. Arch Otolaryngol Head Neck Surg 1997;123(1):34–40 Dulguerov P, Allal AS, Calcaterra TC. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2001;2(11):683–690 Levine PA, Gallagher R, Cantrell RW. Esthesioneuroblastoma: reflections of a 21-year experience. Laryngoscope 1999;109(10): 1539–1543 Nishimura H, Ogino T, Kawashima M, et al. Proton-beam therapy for olfactory neuroblastoma. Int J Radiat Oncol Biol Phys 2007; 68(3):758–762 Simon JH, Zhen W, McCulloch TM, et al. Esthesioneuroblastoma: the University of Iowa experience 1978-1998. Laryngoscope 2001; 111(3):488–493
145
14 15
16
17
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19
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Journal of Neurological Surgery—Part B
Vol. 74
No. B3/2013
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All patients in our series had preserved vision and extraocular motion after completion of treatment. The incidence of reported epiphora was equal in both groups.
Herr et al.
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![[Esthesioneuroblastoma with orbital invasion].](/assets/img/hold.png)
